Ketogenesis and ketolysis (WP4742)
Homo sapiens
This pathway depicts several metabolic pathways involved in ketogenic diet treatment. In hepatocytes in the liver, fatty acids (FAs) are normally transformed into acetyl-CoA, which can then enters the TCA (Krebs) cycle for energy production. However, when FA levels are to high for the TCA cycle to be utilized completely, acetyl-CoA is used in ketogenesis and ketolysis. These processes are a complex process of transporter proteins and several other (mitochondrial) pathways [https://www.ncbi.nlm.nih.gov/books/NBK98219/figure/masino.f1/].
Authors
Raquel Grosman , Egon Willighagen , Kristina Hanspers , Denise Slenter , Eric Weitz , Finterly Hu , and Sam DrabbeActivity
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Organisms
Homo sapiensCommunities
Annotations
Cell Type Ontology
hepatocyte neuron glial cellPathway Ontology
ketone bodies metabolic pathway ketone bodies degradation pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| ATP | Metabolite | chebi:15422 | |
| Fatty acids (FA) | Metabolite | wikidata:Q61476 | |
| BHB | Metabolite | chebi:37054 | BHB aka β-hydroxybutyrate |
| acetone | Metabolite | chebi:15347 | |
| acetone | Metabolite | chebi:15347 | |
| ACA | Metabolite | chebi:15344 | |
| ACA | Metabolite | chebi:15344 | ACA aka Acetoacetate |
| ACA | Metabolite | chebi:15344 | |
| Acetyl-CoA | Metabolite | chebi:15351 | |
| glucose | Metabolite | chebi:17234 | |
| glucose | Metabolite | chebi:17234 | |
| glucose | Metabolite | chebi:17234 | |
| Acetoacetyl-CoA | Metabolite | chebi:15345 | |
| pyruvate | Metabolite | chebi:15361 | |
| pyruvate | Metabolite | chebi:15361 | |
| Acetyl-CoA | Metabolite | chebi:15351 | |
| CO2 | Metabolite | chebi:16526 | |
| Oxaloacetate | Metabolite | chebi:30744 | |
| acetone | Metabolite | chebi:15347 | |
| FA | Metabolite | wikidata:Q61476 | |
| BHB | Metabolite | chebi:37054 | BHB aka β-hydroxybutyrate |
| BHB | Metabolite | chebi:37054 | BHB aka β-hydroxybutyrate |
| BHB | Metabolite | chebi:37054 | BHB aka β-hydroxybutyrate |
| glucose | Metabolite | chebi:17234 | |
| FA | Metabolite | wikidata:Q61476 | |
| CPT-1 | Protein | uniprot:P23786 | CPT1 (mitochondrial isoform) aka carnitine palmitoyl transferase |
| OXCT1 | Protein | uniprot:P55809 | AKA succinyl-CoA-3-oxaloacid CoA transferase (SCOT) |
| CAT | Protein | uniprot:O43772 | CAT aka carnitine translocase |
| BDH1 | Protein | uniprot:Q02338 | AKA 3-hydroxybutyrate dehydrogenase |
| MCT-1 | Protein | uniprot:P53985 | Gene: SLC16A1 |
| UCP2 | Protein | uniprot:P55851 | Mitochondrial transporter proteins which creates proton leaks across the inner mitochondrial membrane, allowing uncoupling oxidative phosphorylation from ATP synthesis. As a result, energy is dissipated in the form of heat. Source [https://www.uniprot.org/uniprot/P55851] |
| GLUT-1 | Protein | uniprot:P11166 | Gene: SLC2A1 |
| ACAT1 | Protein | uniprot:P24752 | mitochondrial acetoacetyl-CoA thiolase |
References
- Jasper’s Basic Mechanisms of the Epilepsies [Internet]. Noebels JL, Avoli M. OUP USA; 2012. 1258 p. Available from: https://books.google.com/books/about/Jasper_s_Basic_Mechanisms_of_the_Epileps.html?hl=&id=T2_LVTB7ftgC OpenLibrary Worldcat
- Structure, function, and regulation of the mammalian facilitative glucose transporter gene family. Olson AL, Pessin JE. Annu Rev Nutr. 1996;16:235–56. PubMed Europe PMC Scholia
- β-Hydroxybutyrate: A Signaling Metabolite. Newman JC, Verdin E. Annu Rev Nutr. 2017 Aug 21;37:51–76. PubMed Europe PMC Scholia
- Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency. Sharpe AJ, McKenzie M. Cells. 2018 May 23;7(6):46. PubMed Europe PMC Scholia